The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins

Roland Lill, Kerstin Diekert, Anita Kaut, Heike Lange, Winfried Pelzer, Corinna Prohl, G. Kispál

Research output: Contribution to journalArticle

115 Citations (Scopus)

Abstract

Iron-sulfur (Fe/S) proteins play an important role in electron transfer processes and in various enzymatic reactions. In eukaryotic cells, known Fe/S proteins are localised in mitochondria, the cytosol and the nucleus. The biogenesis of these proteins has only recently become the focus of investigations. Mitochondria are the major site of Fe/S cluster biosynthesis in the cell. The organelles contain an Fe/S cluster biosynthesis apparatus that resembles that of prokaryotic cells. This apparatus consists of some ten proteins including a cysteine desulfurase producing elemental sulfur for biogenesis, a ferredoxin involved in reduction, and two chaperones. The mitochondrial Fe/S cluster synthesis apparatus not only assembles mitochondrial Fe/S proteins, but also initiates formation of extra-mitochondrial Fe/S proteins. This involves the export of sulfur and possibly iron from mitochondria to the cytosol, a reaction performed by the ABC transporter Atm1p of the mitochondrial inner membrane. A possible substrate of Atm1p is an Fe/S cluster that may be stabilised for transport. Constituents of the cytosol involved in the incorporation of the Fe/S cluster into apoproteins have not been described yet. Many of the mitochondrial proteins involved in Fe/S cluster formation are essential, illustrating the central importance of Fe/S proteins for life. Defects in Fe/S protein biogenesis are associated with the abnormal accumulation of iron within mitochondria and are the cause of an iron storage disease.

Original languageEnglish
Pages (from-to)1157-1166
Number of pages10
JournalBiological Chemistry
Volume380
Issue number10
DOIs
Publication statusPublished - 1999

Fingerprint

Iron-Sulfur Proteins
Mitochondria
Protein S
Iron
Sulfur
Cytosol
Biosynthesis
Prokaryotic Cells
Ferredoxins
Apoproteins
ATP-Binding Cassette Transporters
Hemochromatosis
Mitochondrial Proteins
Mitochondrial Membranes
Eukaryotic Cells
Organelles
Proteins
Electrons
Membranes
Defects

Keywords

  • ABC transporter
  • Chaperone
  • Ferredoxin
  • Iron-sulfur cluster
  • Mitochondria
  • Sideroblastic anemia

ASJC Scopus subject areas

  • Biochemistry

Cite this

The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins. / Lill, Roland; Diekert, Kerstin; Kaut, Anita; Lange, Heike; Pelzer, Winfried; Prohl, Corinna; Kispál, G.

In: Biological Chemistry, Vol. 380, No. 10, 1999, p. 1157-1166.

Research output: Contribution to journalArticle

Lill, R, Diekert, K, Kaut, A, Lange, H, Pelzer, W, Prohl, C & Kispál, G 1999, 'The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins', Biological Chemistry, vol. 380, no. 10, pp. 1157-1166. https://doi.org/10.1515/BC.1999.147
Lill, Roland ; Diekert, Kerstin ; Kaut, Anita ; Lange, Heike ; Pelzer, Winfried ; Prohl, Corinna ; Kispál, G. / The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins. In: Biological Chemistry. 1999 ; Vol. 380, No. 10. pp. 1157-1166.
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